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Liver Cirrhosis Due to an Isolated Inferior Vena Cava Stenosis Causing Outflow Obstruction and Treated by Endovascular Intervention: 2324

The American Journal of Gastroenterology(2018)

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Abstract
Introduction: Budd-Chiari syndrome (BCS) is a syndrome of hepatic venous outflow obstruction that is not due to cardiac or pericardial disease. The increase in hepatic sinusoidal pressure can lead to portal hypertension and related clinical sequelae. The majority of patients with BCS have subacute or chronic presentation, where they can be asymptomatic or present with manifestation of cirrhosis or portal hypertension. In general, isolated IVC blockage in the non-Asian population is rare. Case summary: A 32-year-old Caucasian male with a history of deep vein thrombosis managed on anticoagulation. He was referred over concern for hepatic lesions on abdominal imaging in the setting of unexplained cirrhosis. His symptoms started a few months prior to his referral with shortness of breath and fluid overload. Workup for chronic liver disease was negative, thus, this was labelled cryptogenic cirrhosis. CT and MRI of the liver showed a cirrhotic liver with evidence of portal hypertension, but no focal hepatic lesions. He underwent a liver biopsy which showed cirrhosis with sinusoidal dilation, centrilobular fibrosis and mild chronic portal inflammation raising the concern for an outflow tract obstruction. His cross-sectional images didn't show evidence of hepatic venous obstruction. An echocardiogram revealed a normal ejection fraction and a small atrial septal defect (ASD). The estimated pulmonary artery pressure was 30 mmHg. He was evaluated by the structural heart disease service and the decision was to do a trans-catheter ASD closure as he may undergo transjugular intrahepatic portosystemic shunt or liver transplantation. During the procedure, he was noted to have a severe IVC stenosis and significant gradient between the IVC and the right atrium junction. Angioplasty was performed, and his gradient reduced from 17mmHg to 6mmHg with 18mm balloon dilation. A stent was not placed as this may complicate a future liver transplantation. He improved symptomatically, and his liver and kidney function improved post-procedure. He continues to follow in the clinic for his cirrhosis. Discussion: Among reported cases of BCS, isolated IVC lesions are rare. When present, the most common lesion of the IVC is a segmental occlusion, notably membranous stenosis, as is suspected in this case.Treatment of BCS includes medical, surgical and endovascular options. Endovascular interventions, including balloon angioplasty and stenting, are considered the main therapy for BCS
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Key words
liver cirrhosis,endovascular intervention
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